Related Experiment Video

Updated: Jun 8, 2026

Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models
08:57

Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models

Published on: May 17, 2024

Paraneoplastic neurological disorders

Franz Blaes1, Marlene Tschernatsch

  • 1Department of Neurology, Justus-Liebig-University, Am Steg 14, 35385 Giessen, Germany. Franz.Blaes@neuro.med.uni-giessen.de

Expert Review of Neurotherapeutics
|October 8, 2010
PubMed

Insights

Paraneoplastic neurological disorders (PNDs) involve nervous system dysfunction in cancer patients, often linked to specific autoantibodies. Treatment effectiveness varies based on PND type and antibody targets, with some responding to immunotherapy and others requiring tumor treatment.

Area of Science:

  • Neurology
  • Oncology
  • Immunology

Background:

  • Paraneoplastic neurological disorders (PNDs) are nervous system dysfunctions in cancer patients not caused by direct tumor effects.
  • PNDs are frequently associated with lung cancer, lymphoma, and gynecological tumors.
  • Overlapping clinical syndromes can occur within PNDs.

Purpose of the Study:

  • To provide an overview of the diagnosis and pathogenesis of PNDs.
  • To discuss current therapeutic strategies for patients with PNDs.
  • To explore the autoimmune pathophysiology of PNDs.

Main Methods:

  • Review of existing literature on PND diagnosis, pathogenesis, and treatment.
  • Analysis of the role of autoantibodies in PND development.
  • Categorization of PNDs based on affected neurological sites and associated autoantibodies.

Main Results:

  • PNDs are hypothesized to have an autoimmune pathophysiology, driven by autoantibodies against onconeuronal antigens.
  • Central nervous system PNDs can be T-cell-mediated or caused by receptor autoantibodies (e.g., limbic encephalitis).
  • Neuromuscular junction PNDs and autonomic neuropathies involve receptor or ion channel autoantibodies.

Conclusions:

  • Childhood opsoclonus-myoclonus syndrome and PNDs with receptor/ion channel autoantibodies may respond to immunosuppression, plasmapheresis, and IVIg.
  • Central nervous system PNDs associated with intracellular antigen autoantibodies often require tumor treatment for stabilization.
  • Understanding the specific autoantibody targets is crucial for guiding PND treatment strategies.

More Related Videos

Genetic Profiling and Genome-Scale Dropout Screening to Identify Therapeutic Targets in Mouse Models of Malignant Peripheral Nerve Sheath Tumor09:33

Genetic Profiling and Genome-Scale Dropout Screening to Identify Therapeutic Targets in Mouse Models of Malignant Peripheral Nerve Sheath Tumor

Published on: August 25, 2023

Related Concept Videos

Disorders of the Nervous Tissue01:28

Disorders of the Nervous Tissue

Nervous tissue is a vital component of the human body's communication system, enabling us to perceive and respond to stimuli. However, like all other tissues, it is vulnerable to disorders and diseases that can significantly impact our neurological functioning.
Homeostatic Imbalances:
Alzheimer's disease manifests as a gradual decline in memory and cognitive abilities, attributed to the buildup of amyloid plaques and neurofibrillary tangles in the brain.
Parkinson's disease arises from the...
Parkinson's Disease: Overview01:15

Parkinson's Disease: Overview

Neurodegenerative disorders are progressive diseases that cause irreversible damage and loss to neurons in specific brain areas. Examples of these disorders include Parkinson's disease, Alzheimer's disease, Multiple Sclerosis (MS), and Amyotrophic Lateral Sclerosis (ALS). These disorders share characteristics such as proteinopathies, selective neuronal vulnerability, and a complex interplay between genetic and environmental factors. The primary therapeutic goal for these conditions is to...
Neural Regulation01:37

Neural Regulation

Digestion begins with a cephalic phase that prepares the digestive system to receive food. When our brain processes visual or olfactory information about food, it triggers impulses in the cranial nerves innervating the salivary glands and stomach to prepare for food.
Alterations in Muscle Tone ll01:12

Alterations in Muscle Tone ll

Alterations in muscle tone are common manifestations of neurological disorders and reflect dysfunction within different nervous system regions. Spasticity, paratonia, and dystonia represent distinct forms of hypertonia, each with unique mechanisms, clinical features, and diagnostic importance.CharacteristicsSpasticity happens from upper motor neuron lesions and is characterized by velocity-dependent resistance to passive movement. Clinical features include:Exaggerated deep tendon reflexesClonus...
Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
Cushing Syndrome II: Pathophysiology01:19

Cushing Syndrome II: Pathophysiology

Cortisol production is normally governed by the hypothalamic–pituitary–adrenal (HPA) axis, which maintains hormonal balance through tightly regulated feedback mechanisms. Disruption of this regulatory system is central to the development of Cushing syndrome, whether the excess cortisol originates from external medications or internal pathology. Persistent cortisol elevation alters metabolism, immune function, and endocrine signaling, producing the characteristic clinical features of the...